A multidimensional index and staging system for idiopathic pulmonary fibrosis

Brett Ley, Christopher J. Ryerson, Eric Vittinghoff, Jay H Ryu, Sara Tomassetti, Joyce S. Lee, Venerino Poletti, Matteo Buccioli, Brett M. Elicker, Kirk D. Jones, Talmadge E. King, Harold R. Collard

Research output: Contribution to journalArticle

439 Citations (Scopus)

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. Design: A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Setting: Interstitial lung disease referral centers in California, Minnesota, and Italy. Patients: 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). Measurements: The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Results: Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and DLCO). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9). Limitation: Patients were drawn from academic centers and analyzed retrospectively. Conclusion: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF. Primary Funding Source: University of California, San Francisco Clinical and Translational Science Institute.

Original languageEnglish (US)
Pages (from-to)684-695
Number of pages12
JournalAnnals of Internal Medicine
Volume156
Issue number10
StatePublished - 2012

Fingerprint

Idiopathic Pulmonary Fibrosis
San Francisco
Mortality
Interstitial Lung Diseases
Italy
Calibration
Lung Diseases
Referral and Consultation
Transplantation
Lung
Research

ASJC Scopus subject areas

  • Internal Medicine

Cite this

Ley, B., Ryerson, C. J., Vittinghoff, E., Ryu, J. H., Tomassetti, S., Lee, J. S., ... Collard, H. R. (2012). A multidimensional index and staging system for idiopathic pulmonary fibrosis. Annals of Internal Medicine, 156(10), 684-695.

A multidimensional index and staging system for idiopathic pulmonary fibrosis. / Ley, Brett; Ryerson, Christopher J.; Vittinghoff, Eric; Ryu, Jay H; Tomassetti, Sara; Lee, Joyce S.; Poletti, Venerino; Buccioli, Matteo; Elicker, Brett M.; Jones, Kirk D.; King, Talmadge E.; Collard, Harold R.

In: Annals of Internal Medicine, Vol. 156, No. 10, 2012, p. 684-695.

Research output: Contribution to journalArticle

Ley, B, Ryerson, CJ, Vittinghoff, E, Ryu, JH, Tomassetti, S, Lee, JS, Poletti, V, Buccioli, M, Elicker, BM, Jones, KD, King, TE & Collard, HR 2012, 'A multidimensional index and staging system for idiopathic pulmonary fibrosis', Annals of Internal Medicine, vol. 156, no. 10, pp. 684-695.
Ley B, Ryerson CJ, Vittinghoff E, Ryu JH, Tomassetti S, Lee JS et al. A multidimensional index and staging system for idiopathic pulmonary fibrosis. Annals of Internal Medicine. 2012;156(10):684-695.
Ley, Brett ; Ryerson, Christopher J. ; Vittinghoff, Eric ; Ryu, Jay H ; Tomassetti, Sara ; Lee, Joyce S. ; Poletti, Venerino ; Buccioli, Matteo ; Elicker, Brett M. ; Jones, Kirk D. ; King, Talmadge E. ; Collard, Harold R. / A multidimensional index and staging system for idiopathic pulmonary fibrosis. In: Annals of Internal Medicine. 2012 ; Vol. 156, No. 10. pp. 684-695.
@article{cda9c0c87d7146c0adfd2500836926be,
title = "A multidimensional index and staging system for idiopathic pulmonary fibrosis",
abstract = "Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. Design: A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Setting: Interstitial lung disease referral centers in California, Minnesota, and Italy. Patients: 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). Measurements: The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Results: Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and DLCO). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6{\%}, 16{\%}, and 39{\%}, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9). Limitation: Patients were drawn from academic centers and analyzed retrospectively. Conclusion: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF. Primary Funding Source: University of California, San Francisco Clinical and Translational Science Institute.",
author = "Brett Ley and Ryerson, {Christopher J.} and Eric Vittinghoff and Ryu, {Jay H} and Sara Tomassetti and Lee, {Joyce S.} and Venerino Poletti and Matteo Buccioli and Elicker, {Brett M.} and Jones, {Kirk D.} and King, {Talmadge E.} and Collard, {Harold R.}",
year = "2012",
language = "English (US)",
volume = "156",
pages = "684--695",
journal = "Annals of Internal Medicine",
issn = "0003-4819",
publisher = "American College of Physicians",
number = "10",

}

TY - JOUR

T1 - A multidimensional index and staging system for idiopathic pulmonary fibrosis

AU - Ley, Brett

AU - Ryerson, Christopher J.

AU - Vittinghoff, Eric

AU - Ryu, Jay H

AU - Tomassetti, Sara

AU - Lee, Joyce S.

AU - Poletti, Venerino

AU - Buccioli, Matteo

AU - Elicker, Brett M.

AU - Jones, Kirk D.

AU - King, Talmadge E.

AU - Collard, Harold R.

PY - 2012

Y1 - 2012

N2 - Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. Design: A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Setting: Interstitial lung disease referral centers in California, Minnesota, and Italy. Patients: 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). Measurements: The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Results: Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and DLCO). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9). Limitation: Patients were drawn from academic centers and analyzed retrospectively. Conclusion: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF. Primary Funding Source: University of California, San Francisco Clinical and Translational Science Institute.

AB - Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with an overall poor prognosis. A simple-to-use staging system for IPF may improve prognostication, help guide management, and facilitate research. Objective: To develop a multidimensional prognostic staging system for IPF by using commonly measured clinical and physiologic variables. Design: A clinical prediction model was developed and validated by using retrospective data from 3 large, geographically distinct cohorts. Setting: Interstitial lung disease referral centers in California, Minnesota, and Italy. Patients: 228 patients with IPF at the University of California, San Francisco (derivation cohort), and 330 patients at the Mayo Clinic and Morgagni-Pierantoni Hospital (validation cohort). Measurements: The primary outcome was mortality, treating transplantation as a competing risk. Model discrimination was assessed by the c-index, and calibration was assessed by comparing predicted and observed cumulative mortality at 1, 2, and 3 years. Results: Four variables were included in the final model: gender (G), age (A), and 2 lung physiology variables (P) (FVC and DLCO). A model using continuous predictors (GAP calculator) and a simple point-scoring system (GAP index) performed similarly in derivation (c-index of 70.8 and 69.3, respectively) and validation (c-index of 69.1 and 68.7, respectively). Three stages (stages I, II, and III) were identified based on the GAP index with 1-year mortality of 6%, 16%, and 39%, respectively. The GAP models performed similarly in pooled follow-up visits (c-index ≥71.9). Limitation: Patients were drawn from academic centers and analyzed retrospectively. Conclusion: The GAP models use commonly measured clinical and physiologic variables to predict mortality in patients with IPF. Primary Funding Source: University of California, San Francisco Clinical and Translational Science Institute.

UR - http://www.scopus.com/inward/record.url?scp=84861162888&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84861162888&partnerID=8YFLogxK

M3 - Article

C2 - 22586007

AN - SCOPUS:84861162888

VL - 156

SP - 684

EP - 695

JO - Annals of Internal Medicine

JF - Annals of Internal Medicine

SN - 0003-4819

IS - 10

ER -